Edit concept Question Editor Create issue ticket

Congenital Pseudoarthrosis of the Tibia


Presentation

  • The objective is to evaluate the present status and future role of free vascular fibular transfer in treating congenital pseudoarthrosis of the tibia.[ncbi.nlm.nih.gov]
  • Four patients presented femur overgrowth. The average shortening of the affected leg was 3.6 cm. The proposed procedure leads to a long treatment course with many reoperations for correction of possible complications.[ncbi.nlm.nih.gov]
  • CASE REPORT A 12-year-old boy presented with a bowed leg. X-ray tibia showed partially healed congenital pseudoarthrosis of the tibia and 30 degrees of flexion deformity.[ncbi.nlm.nih.gov]
  • Reports described clinical presentation to range from simple anterolateral bowing of tibia to frank pseudarthrosis 3 .In this series 25 out of 37(60%) presented with established pseudarthrosis, while 10 out of 37(27%) presented with bowing.[sudjms.net]
  • Presenting problem: The table summarises patient characteristics, presenting problem, safeguarding assessment and action taken by social services. Café-au-lait lesions were present in all 3 patients.[bone-abstracts.org]
Surgical Procedure
  • Numerous surgical procedures have been used including bone grafting, fixation, pulsed electromagnetic field, vascularized fibular bone grafting and lengthening devices.[ncbi.nlm.nih.gov]
  • The average follow-up was 16 years from the first and 8 years from the last surgical procedure.[ncbi.nlm.nih.gov]
  • The achievement of a healed, straight extremity by skeletal maturity, with as few surgical procedures as possible, is essential for a child with congenital pseudarthrosis of the tibia.[journals.lww.com]
  • Vascularized fibular grafts may be indicated for pseudarthroses with gaps of more than 3 cm and for pseudarthroses in which multiple surgical procedures have failed.[orthopedicnotes.blogspot.com]
  • Hence, patients may request amputation despite the fact that union has finally been gained.2'2 Early amputation as a definitive treatment may avoid numerous surgical procedures and a lifetime of disability from casts, braces, and other aspects of treatment[healio.com]
Noncompliance
  • […] significantly higher with a younger age at surgery (less than 4 years), low cross-sectional area of the healed segment, recurrence of tumor, when associated with persistent fibular pseudarthrosis, residual ankle valgus, removal of an intramedullary rod, and noncompliance[ijoonline.com]
Leg Swelling
  • On clinical examination, the child was found to have multiple café-au-lait spots, axillary freckling, and right leg swelling [Figure 1].[saudijhealthsci.org]
Lymphedema
  • […] tibia Congenital undergrowth of lower limb Congenital undergrowth of partial lower limb Congenital undergrowth of whole of lower limb Congenital valgus ankle Congenital varus ankle Constriction ring of lower limb Constriction ring of lower limb with lymphedema[icd9data.com]
Italian
  • Mastragostino", year "1996", language "Italian", volume "47", pages "441--446", journal "Minerva Ortopedica", issn "0026-4911", publisher "Edizioni Minerva Medica S.p.A.", number "9", } TY - JOUR T1 - Pseudoartrosi congenita della gamba.[moh-it.pure.elsevier.com]
Joint Stiffness
  • Clinically, the following parameters were assessed: pain, joint stiffness, and limb length discrepancy (LLD).[ncbi.nlm.nih.gov]
  • Treatment is mainly surgical and it aims to obtain a long term bone union, to prevent limb length discrepancies, to avoid mechanical axis deviation, soft tissue lesions, nearby joint stiffness, and pathological fracture.[scienceopen.com]
  • Treatment is mainly surgical and it aims to obtain a long term bone union, to prevent limb length discrepancies, to avoid mechanical axis deviation, soft tissue lesions, nearby joint stiffness, and pathological fracture...[readbyqxmd.com]
Recurrent Fractures
  • However, surgical intervention is required in case of non-union and recurrent fractures. The surgical therapy consists of resection of the diseased segment and bone grafting, followed by internal or external fixation [ 3 ].[e-aps.org]
Leg Length Inequality
  • inequality commonly develops that often requires treatment. [6] If a significant leg-length inequality results, the patient will have an abnormal gait and may be at risk for increased back pain or deformity.[emedicine.medscape.com]
Suggestibility
  • Several evidences suggest that decreased osteogenic capacities, impaired local vascularization, and microenvironment alterations may play a role in the pathogenesis of CPT.[ncbi.nlm.nih.gov]
  • CONCLUSION: In a five year follow-up, these results suggest that rh-BMP-7 and autologous bone grafting is no better than autologous grafting alone.[ncbi.nlm.nih.gov]
  • This result suggests that CPT is not accompanied by double inactivation in every NF1 case.[link.springer.com]
  • Suggested causes include abnormal fetal position or intrauterine fracture, or a primary defect in the embryological development of the lower leg and the tibial or fibular shafts. · Prognosis is good.[fetalultrasound.com]
  • Also noted are abnormal dilated vascular channels suggestive of collateral formation (arrows in c) Click here to view Figure 4: Duplex ultrasound image.[saudijhealthsci.org]

Treatment

  • At 6 months after the treatment, a small amount of bridging callus was seen. We continued the treatment for 1 year until the solid fusion was observed on radiographs and the patient started full-weight-bearing.[ncbi.nlm.nih.gov]
  • The union rate was 94% with one treatment and 100% with two treatments. There were five refractures, three early and two late. Previous pin sites, residual angular deformity, and natural history were considered predisposing factors for refractures.[ncbi.nlm.nih.gov]
  • Conventional treatments used included excision of the pseudoarthrosis, correction of the deformity, intramedullary fixation, bone grafting and soft tissue transplantation for lengthening and coverage.[ncbi.nlm.nih.gov]
  • The proposed procedure leads to a long treatment course with many reoperations for correction of possible complications.[ncbi.nlm.nih.gov]
  • In 250 patients with tibial surgical treatment, the highest rate of fusion occurred in patients with fibular involvement compared with those with normal fibula.[ncbi.nlm.nih.gov]

Prognosis

  • The prognosis of CPT is very much related to the radiologic classification. Crawford type 2 and Crawford type 4 pseudoarthrosis have a worse prognosis, with a lower percentage of fusion at the site of pseudoarthrosis.[ncbi.nlm.nih.gov]
  • Prognosis continues to be reserved especially in clepsydra forms and those localised to the lower third of the tibia.[moh-it.pure.elsevier.com]
  • Pseudarthrosis develops through a congenital cyst Type IV : Pseudarthrosis develops in a sclerotic segment of bone without narrowing, stress fracture Type V : Pseudarthrosis of the tibia occurs with a dysplastic fibula, pseudarthrosis of one or both bones (prognosis[pseudarthrose.ch]
  • Type 2 have a relative poor prognosis. Type 3 cases are usually of late onset type which develops as a stress fracture of congenital dysplastic tibia or in a healed tibia after previous bone grafting & has the best prognosis.[de.slideshare.net]
  • Prognosis worse with Neurofibromatosis 1/2 of cases 3 Fracture develops at site of bone cyst Usually near junction of mid/ distal 1/3 Anterior bowing precedes or follow fracture 4 Sclerotic bony segment initially present at junction of mid/ distal 1[orthofracs.com]

Etiology

  • Congenital pseudoarthrosis of the tibia (CPT) is an uncommon disease whose etiology and pathogenesis is unknown.[ncbi.nlm.nih.gov]
  • Pathology The etiology is unclear, however, around 50% of cases are associated with neurofibromatosis type 1 (NF1). Overall, 10% of patients with NF1 are diagnosed with tibial pseudoarthrosis.[radiopaedia.org]
  • Abstract The purpose of study was to refine frequency of etiological factors and characteristics of congenital pseudarthrosis of tibia (CPT). Materials and Methods.[journals.eco-vector.com]
  • Etiology Each type of tibial bowing tends to have its own etiology.[emedicine.medscape.com]
  • The etiological nature of this lesion is unclear, however there is a strong association between CPT and neurofibromatosis type I (NF-I).[jlponline.org]

Epidemiology

  • Horn J, Steen H, Terjesen T (2013) Epidemiology and treatment outcome of congenital pseudarthrosis of the tibia. J Child Orthop 7(2):157–166 PubMedCentral CrossRef PubMed 20.[springermedizin.de]
  • Epidemiological data are limited, and treatment of the condition is challenging. The purpose of our study was to gain epidemiological data on the incidence of CPT in Norway and to evaluate the treatment outcome of the disease.[readbyqxmd.com]
  • Bowing usually at junction of Mid / Distal 1/3 Epidemiology Rare 1:200 000 M F 50% have Neurofibromatosis type 1 1-3% of Neurofibromatosis have CPT Aetiology Cause unknown Theories include: Intrauterine fracture Localised vascular abnormality Constriction[orthofracs.com]
  • Hefti, F, Bollini, G, Dungl, P, Fixsen, J, Grill, F, Ippolito, E, et al. 2000 Congenital pseudarthrosis of the tibia: history, etiology, classification, and epidemiologic data J Pediatr Orthop B 9 11 5 PubMed Google Scholar 16.[link.springer.com]
  • Congenital pseudoarthrosis of the tibia: History, etiology, classification and epidemiologic data. J Pediatr Orthop B 2000;9:11-5. [ PUBMED ] 2. Andersen KS, Bohr H, Sheppen O. Congenital angulation of the lower leg. Crus curvatum congenitum.[jlponline.org]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology, evaluation, and treatment. J Bone Joint Surg Am 2005;87:1848-64. Wang K, Allen L, Fung E, et al. Bone scintigraphy in common tumors with osteolytic components. Clin Nucl Med 2005;30:655-71. Tuncel M, Kiratli PO, Gedikoglu G.[journals.sfu.ca]
  • A relationship with type 1 neurofibromatosis or von Recklinghausen’s disease is known but exact pathophysiology has still not been clearly defined.[boneandspine.com]
  • Appropriate interpretation will improve understanding of the natural history and pathophysiology of neurofibromatosis type 1.[nature.com]
  • The pathophysiology of the resultant limb-length inequality may be related to the bowing.[emedicine.medscape.com]

Prevention

  • At the end of the correction, a telescopic Fassier-Duval intramedullary nail was used prophylactically to prevent re-fracture. After 2 years of follow-up, X-ray images show bone-healing without any bowing.[ncbi.nlm.nih.gov]
  • Moreover it does not always prevent ankle valgus [Figure 4]. To prevent all these, the rod must be kept till skeletal maturity.[ijoonline.com]
  • Created to prevent or stabilize fractures, or correct deformity of long bones whilst growth occurs.[pegamedical.com]
  • , poor protection against re-fracture, failure to correct limb length discrepancy, and deformities of leg and ankle simultaneously at time of primary surgery. 8 Vascularized fibular graft/ Nail The intramedullary rod offers good tibial alignment and prevents[slideplayer.com]

Ask Question

5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.
By publishing this question you agree to the TOS and Privacy policy.
• Use a precise title for your question.
• Ask a specific question and provide age, sex, symptoms, type and duration of treatment.
• Respect your own and other people's privacy, never post full names or contact information.
• Inappropriate questions will be deleted.
• In urgent cases contact a physician, visit a hospital or call an emergency service!